Sickle Cell Disease

Question 1

What bugs are we worried about with sickle cell?

What vaccines are important in SCD?

What is non drug treatment of SCD?

Answer 1

Strep pneumo, Haemophilus, Neisseria.

Haemophilus B, PCV13 and Prevnar, Meningococcal conjugate series, pneumococcal pneumovax and pcv13 in any patient older than 6 if never received as part of a routine childhood series,.

Blood transfusions, the only cure is bone marrow transplantation, chelation therapy, Hgb should be no higher than 10 g/dL.

Question 2

What to know about hydroxyurea?

Answer 2

Stimulates production of HgbF. BW of myelosuppresion. Embryo-fetal toxicity, avoid live vaccinations, CBC with diff, ANC, contraception required, hazardous agent, Folic Acid. Wear sun protection.